OBJECTIVES Jeune syndrome (asphyxiating thoracic dystrophy) is a rare disorder characterized by skeletal dysplasia, reduced diameter of the thoracic cage and extrathoracic organ involvement. Fatal, early respiratory insufficiency may occur. Two-stage lateral thoracic expansion has been reported, addressing each side sequentially over 3-12 months. While staged repair theoretically provides less ...

allgrove syndrome also known as triple-a syndrome is an autosomal recessive disorder characterized by alacremia, achalasia and acth-resistant adrenal insufficiency. although this syndrome is rare, herein we report four cases with different clinical manifestations. they were referred to the gastrointestinal ward during a one year period with complaints of vomiting and dysphagia. the diagnosis of...

Affiliations: Service des Soins Intensifs et Urgences Oncologiques, Oncologie Thoracique, Institut Jules Bordet, Brussels, Belgium. Educational Activities Dept, European Respiratory Society, Lausanne, Switzerland. Division of Respiratory Medicine, Klinikum Straubing GmbH, Straubing, Germany. For a list of the Thoracic Oncology HERMES Task Force members and their affiliations see the Acknowledge...

Background Given the relationship of vitamin D deficiency with insulin resistance syndrome as the component of polycystic ovary syndrome (PCOS), the main aim of this study was to compare serum level of 25- hydroxyvitamin D [25(OH)D] between PCOS patients and normal individuals. MaterialsAndMethods A cross sectional study was conducted to compare 25(OH)D level between117 normal and 125 untreated...

Background Early-onset scoliosis, when left untreated, leads to severe deformity. Until the last decade, treatment of progressive curves in young children often consisted of definitive spinal fusion. The recognition of thoracic insufficiency syndrome associated with definitive early fusion has led to the development of new surgical techniques developed to preserve spinal and thoracic growth in ...

Review of 10 cases of asphyxiating thoracic dysplasia has shown a wide range of clinical effects and some variability in the radiographic features. Respiratory difficulty was severe in 7 babies and lethal in 6 of these. The seventh child is remarkable for his normal stature and excellent health at 15 years of age. 3 babies had no respiratory difficulty but 2 of them subsequently died of renal f...

Surgery to replace the aortic root with a valved tube, whether mechanical or biological, remains most widely used technique for correcting diseases affecting this segment of aorta. Although valves are usually used, they expose patients risk thromboembolic complications associated anticoagulation. We report case first Tirone David operation performed at Festoc centre in Bamako. The patient was 6...

a 32-year-old female patient with previous bentall operation and mitral valve repair surgery due to severe aortic insufficiency, mitral valve insufficiency, and ascending aortic aneurysm was admitted to our hospital with serious dyspnea, fatigue, and mild chest pain. two-dimensional echocardiography demonstrated a markedly dilated basal aorta and cardiac chambers. thoracic computed tomography s...

To cite: Kapoor N, Shetty S, Shetty S, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014203672 DESCRIPTION A 19-year-old boy presented with history of progressive dysphagia and vomiting of 3 years duration. Dysphagia was more for liquids than solids. At the age of 8 he had vomiting and asthenia, and was noticed to have hyperpigmentation. At that time he ...